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Background  In β-thalassemia, profound anemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems. In recent years, there have been few published studies mainly in adult demonstrating renal involvement in β-thalassemia. This prospective study was aimed to investigate renal involvement in pediatric patients with transfusion dependant beta-thalassemia major (TD-βTM), using both conventional and early markers of glomerular and tubular dysfunctions, and to correlate findings to oxidative stress and iron chelation therapy.    Methods  Sixty-nine TD-βTM patients (aged 1-16 years) and 15 healthy controls (aged 3-14 years) were enrolled in this study. Based on receiving chelation therapy (deferoxamine, DFO), patients were divided into two groups: group [I] with chelation (n = 34) and group [II] without chelation (n = 35). Levels of creatinine (Cr), calcium (Ca), inorganic phosphorus (PO 4 ), uric acid (UA) and albumin were measured by spectrophotometer. Serum (S) levels of cystatin-C (S CysC ) and total antioxidant capacity (S TAC ) and urinary (U) levels of β 2 -microglobulin (U β2MG ) were measured by immunosorbent assay (ELISA). Urinary N-acetyl-beta-D-glucosaminidase (U NAG ) activity and malondialdehyde (U MDA ) were measured by chemical methods. Estimated glomerular filtration rate (eGFR) was determined from serum creatinine.    Results  In patient with and without chelation, glomerular [elevated S CysC , S Cr , U albumin /Cr and diminished eGFR]; and tubular dysfunctions [elevated S UA , S PO4 , U NAG /Cr, U β2MG /Cr] and oxidative stress marker disturbances [diminished S TAC  and elevated U MDA /Cr] were reported than controls. In patients with chelation, S CysC  was significantly higher while, S TAC  was significantly lower than those without chelation. In all patients, S CysC  showed significant positive correlation with S Cr  and negative correlation with eGFR; S TAC  showed significant positive correlation with eGFR and negative correlation with S Cys C, S Cr , U NAG /Cr; U MDA /Cr showed significant positive correlation with U albumin /Cr, U β2MG /Cr, U NAG /Cr.    Conclusions  Our data confirm high frequency of glomerular and tubular dysfunctions in TD-βTM pediatric patients which could be attributed to oxidative stress and DFO therapy.

Renal Functions in pediatric patients with beta-thalassemia major: relation to chelation therapy: original prospective study